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2 Frontotemporal DementiaDefinition: clinicopathologic condition consisting of deterioration of personality and cognition assoc. with prominent frontal and temporal lobe atrophyAccounts for up to 3-20% of dementiasThird behind AD and Lewy Body Dementia in neurodegenerative dementing illnesses

3 PrevalenceMean age of onset 52.8 (Ratnavalli et al. Neurology 2002;58: )Male preponderance 14:3 in one study and M=F in othersDementia prevalence of 81 per 100,000 (95% CI, 62.8 to 104.5) in the year age groupPrevalence of AD and FTD in age group same at 15 per 100,000 ( )

10 Frontotemporal DementiaRecent study showed of 42 FTD cases, 19 had at least 1 other family member affected (Chow et al., Arch Neurol. 1999;56: )1/3 had a positive family history in this study, but others much lowerAppears to be inherited in autosomal dominant fashion in those patientsLinked to chromosome 17 (tau gene) and chromosome 3Association with motor neuron disease (ALS)

13 Frontotemporal DementiaTerm is conventionally used in clinical and pathological diagnoses in standard clinical settingNo other specific code comes reasonably close to capturing diseaseDementia or AD diagnosis (290 or 331) ignores established diagnostic criteria and allows no tracking of FTD

14 Frontotemporal DementiaDiffers from the other codes including AD, frontal dementia, Pick’s diseaseUse of these codes inappropriate as not capture the age of onset, duration of illness, genetic factors, and impact on caregiver, society, and economicsAD  older, different duration, less clear geneticsFrontal dementia  no temporal lobe involvement, genetics differPick’s disease  not capture spectrum of FTD

15 Frontotemporal DementiaImplications for FTD different from other dementias/AD:Greater caregiver burden and increased dependency and health care costsPatients see codes and think they have some other disease